Neurofibromatose de Von Recklinghausen

A neurofibromatose é uma doença multissistémica na qual as possíveis múltiplas manifestações clínicas podem ter importantes implicações diagnósticas e prognósticas: se coexiste hipertensão arterial, vários mecanismos patogénicos justificam que se considere a possibilidade da etiologia secundária. Neste trabalho, revemos mecanismos patogénicos e manifestações clínicas, e apontamos estratégias diagnósticas para anomalias e doenças, que embora presentes, podem estar ocultas.

Biópsia per-oral do intestino delgado em 150 casos de diferentes enteroparasitoses

Os autores fazem uma apresentação sumária de alguns tipos de instrumentos utilizados na realização da biópsia duodeno-jejunal através intubação oral, ao mesmo tempo que comentam a importância do método no estudo da patologia intestinal. Em seguida apresentam os resultados de sua experiência empregando 6 diferentes tipos de sondas e mostram os resultados histopatológicos observados em 150 casos de diversas parasitoses intestinais, submetidos a estudo. Entre os resultados chamam a atenção para a presença freqüente da Giardia lamblia, detectada em 16 dos 50 (32%) casos desta parasitose, contra 3 dos 27 (11,1%) de esquistossomose e apenas 2 dos 108 (1,8%) de estrongiloidíase. Relatam também o achado de numerosas leishmânias no córion da mucosa jejunal de um paciente parasitado pela Giardia lamblia, que era portador de Calazar. Apreciam, ainda, o significado de outras alterações, consideradas inespecíficas, sobre as quais aguardam estudos mais completos, para chegar a conclusões mais válidas. Entre estas, merece destaque o achado de atrofia das vilosidades intestinais em 27 pacientes infectados pelo Strongyloides stercoialis tendo que em 5 dêles havia parasitismo exclusivo pelo referido nematóide.

Reporting earnings per share: Evidence from Portuguese annual reports

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Finance from the NOVA – School of Business and Economics

Atherogenesis and atherosclerosis in primary antiphospholipid syndrome

ABSTRACT: In the late seventies the term “Haematological Stress Syndrome” defined some haematological abnormalities appearing in the course of acute and chronic disorders, such as raised plasma levels of fibrinogen (FNG) and factor VIII, reduced fibrinolytic activity and hyperviscosity. In the early nineties the “Membrane stress syndrome hypothesis” proposed the unification of the concepts of haematological stress syndrome with those of oxidation, inflammation and immune activation to explain the pathogenesis of the antiphospholipid syndrome (APS) Antiphospholipid antibodies, coagulation, fibrinolysis and thrombosis. This chapter investigated the occurrence of the “Haematological Stress Syndrome” and thrombosis in 144 participants positive for aPL detected by clotting and immune tests. Among the clotting assays for the detection of lupus anticoagulant, dilute Russell's viper venom time better correlated with a history of venous thrombosis than activated partial thromboplastin time (p<0.0002 vs p<0.009) and was the only test correlated with a history of arterial thrombosis (p<0.01). By regression analysis, serum levels of IgG anticardiolipin antibodies (aCL) associated with the number of venous occlusions (p<0.001). With regards to FNG and von Willebrand factor (vWF), the former rose by 36% (95% CI; 21%, 53%) and the latter by 50% (95% CI; 29%, 75%) at the first venous occlusion and remained unchanged after subsequent occlusions. At variance FNG rose by 45% (95% CI; 31%, 60%) per arterial occlusion and vWF by 27% (95% CI; 10%, 47%) per arterial occlusion throughout. The coagulation/fibrinolytic balance was cross-sectionally evaluated on 18 thrombotic PAPS patients, 18 subjects with persistence of idiopathic aPL and in healthy controls. Markers of thrombin generation prothrombin fragment 1+2 (F1+2), thrombin-antithrombin complex (TAT) and of fibrin turnover D-Dimer (D-D) were higher in thrombotic (p=0.006)and non-thrombotic subjects (p=0.0001) than in controls as were those of D-D (p<0.0001 and p=0.003 respectively). TAT levels did not differ. Gender analysed data revealed blunted tPA release (hence a negative venous occlusion test) in thrombotic females but neither in thrombotic males (p=0.01) nor in asymptomatic subjects of either sex. Also, in both patient groups females had higher mean PAI than males (p<0.0002) and control females (p<0.02). The activity of factor XIII (FXIIIa) was evaluated was evaluated in 29 patients with PAPS, 14 persistent carriers of aPL without thrombosis, 24 thrombotic patients with inherited thrombophilia, 28 healthy controls and 32 patients with mitral and aortic valve prosthesis as controls for FXIII only. FXIIIa was highest in PAPS (p=0.001), particularly in patients with multiple (n=12) than single occlusion (p=0.02) and in correlation with PAI (p=0.003) and FNG (p=0.005). Moreover FXIIIa was strongly associated with IgG aCL and IgG anti-2GPI (p=0.005 for both) in the PAPS group and to a lesser degree in the aPL group (FXIIIa with IgG aCL, p=0.02, with IgG anti-2GPI, p=0.04). Altogether these results indicate: 1) a differential relationship of aPL, vWF and FNG with venous and arterial thrombosis; 2) heightened thrombin generation, accelerated fibrin turnover and fibrinolysis abnormalities also in asymptomatic carriers of aPLs; 3) enhanced FXIIIa that may contribute to atherothrombosis via increased fibrin/fibrinogen cross-linking. Lipid profile, lipid peroxidation and anti-lipoprotein antibodies in thrombotic primary antiphospholipid syndrome. Given the atherogenic lipid profile of SLE, the same possibility was explored in PAPS by comparing high-density lipoprotein (HDL), low-density lipoprotein (LDL), total cholesterol (CHO), apolipoprotein AI (ApoAI), apolipoprotein B (ApoB), triglycerides (TG), anti-lipoprotein antibodies, beta-2-glycoprotein I complexed to oxidized low-density lipoprotein (oxLDL-2GPI) and C-reactive protein (CRP) in 34 thrombotic PAPS patients compared to 36 thrombotic patients with inherited thrombophilia (IT), to 18 subjects persistently positive for antiphospholipid antibodies (aPL) with no underlying autoimmune or non-autoimmune disorders and to 28 healthy controls. Average concentrations of HDL (p<0.0001), LDL (p<0.0001), CHO (p=0.0002), ApoAI (p=0.002) were lower in PAPS whereas average TRY was higher (p=0.01) than other groups. Moreover PAPS showed higher IgG anti-HDL (p=0.01) and IgG anti-ApoAI (p<0.0001) as well as greater average oxLDL-2GPI (p=0.001) and CRP (p=0.003). Within PAPS, IgG anti-HDL correlated negatively to HDL (p=0.004) and was an independent predictor of oxLDL-2GPI (p=0.009). HDL and ApoAI correlated negatively with CRP (p=0.001 and p=0.007, respectively). IgG anti-HDL may hamper the antioxidant and anti-inflammatory effect of HDL favouring low-grade inflammation and enhanced oxidation in thrombotic PAPS. Indeed plasma 8-epi-prostaglandin F2α (a very specific marker of lipid peroxidation) was significantly higher in 10 patients with PAPS than 10 age and sex matched healthy subjects (p=0.0002) and strongly related to the titre of plasma IgG aCL (r=0.89, p=0.0004). Hence oxidative stress, a major player in atherogenesis, also characterises PAPS. Nitric oxide and nitrative stress in thrombotic primary antiphosholipid syndrome. Oxidative stress goes hand in hand with nitrative stress and to address the latter plasma nitrotyrosine (NT, marker of nitrative stress), nitrite (NO2-) and nitrate (NO3-) were measured in 46 thrombotic PAPS patients, 21 asymptomatic but persistent carriers of antiphospholipid antibodies (PCaPL), 38 patients with inherited thrombophilia (IT), 33 patients with systemic lupus erythematosus (SLE) and 29 healthy controls (CTR). Average crude NT was higher in PAPS and SLE (p=0.01) whereas average plasma NO2- was lower in PAPS and average NO3- highest in SLE (p<0.0001). In PAPS, IgG aCL titer and number of vascular occlusions negatively predicted NO2-, (p=0.03 and p=0.001, respectively) whereas arterial occlusions and smoking positively predicted NO3- (p=0.05 and p=0.005). Moreover CRP (an inflammatory marker) positively predicted NT (p=0.004). Nitric oxide metabolites relates to type and number of vascular occlusions and to aPL titers, whereas nitrative stress relates to low grade marker) positively predicted NT (p=0.004). Nitric oxide metabolites relates to type and number of vascular occlusions and to aPL titers, whereas nitrative stress relates to low grade inflammation and both phenomena may have implications for thrombosis and atherosclerosis in PAPS Inflammation and immune activation in thrombotic primary antiphospholipid syndrome. To investigate inflammation and immune activation in thrombotic PAPS high-sensitivity CRP (hs-CRP), serum amyloid A (SAA), oxLDL-2GPI, CRP bound to oxLDL-2GPI (CRP-oxLDL-2GPI) (as inflammatory markers) neopterin (NPT) and soluble CD14 (sCD14) (as immune activation markers) were measured by ELISA in 41 PAPS patients, in 44 patients with inherited thrombophilia (IT) and 39 controls (CTR). Compared to other groups, PAPS presented with higher plasma concentrations of inflammatory, hs-CRP (p=0.0004), SAA (p<0.01), CRP-oxLDL-2GPI (p=0.0004) and immune activation markers, NPT (p<0.0001) and sCD14 (p=0.007). By regression analysis SAA independently predicted thrombosis number (p=0.003) and NPT independently predicted thrombosis type (arterial, p=0.03) and number (p=0.04). These data confirm that low-grade inflammation and immune activation occur and relate to vascular features of PAPS. Antiphosholipid antibodies, haemostatic variables and atherosclerosis in thrombotic primary antiphospholipid syndrome To evaluate whether IgG aCL titre, haemostatic variables and the lipid profile bore any relationship to the intima media thickness (IMT) of carotid arteries high-resolution sonography was applied to the common carotid (CC), carotid bifurcation (CB) and internal carotid (IC) of 42 aPL subjects, 29 with primary thrombotic antiphospholipid syndrome and 13 with persistence of aPL in the absence of any underlying disorder. The following were measured: plasma FNG, vWF, PAI, homocysteine (HC), CHO, TG, HDL, LDL, platelet numbers and aCL of IgG and IgM isotype. By multiple regression analysis, IgG aCL titre independently predicted IMT at all carotid segments examined (p always <0.005). Plasma FNG and HC independently predicted IMT at the CB (p=0.001 and p<0.0001, respectively) and IC (p=0.03 and p<0.0001, respectively). These data strongly support an atherogenic role for IgG aCL in patients with aPL in addition to traditional risk factors. The atherosclerosis hypothesis was investigated in an age and sex-matched case-double-control study including 49 thrombotic PAPS patients (18 M, 31 F, mean age 37 ± 11), 49 thrombotic patients for IT and 49 healthy subjects. Average IMT was always greater in PAPS than control patients (CC: p=0.004, CB: p=0.013, IC: p=0.001). By dividing participants into age tertiles the IMT was greater in the second (CC: p=0.003, CB: p=0.023, IC: p=0.003) and third tertiles (CC: p=0.03, CB: p=0.004, IC: p=0.007). Conclusion: Coagulation activation, fibrinolysis depression, hightened fibrin turnover, oxidative and nitrative stress in parallel with low grade inflammation and immune activation characterise thrombotic PAPS: all these are early atherogenic processes and contribute to the demonstrated premature atherosclerosis that should be considered a clinical feature of PAPS.

Surto de peste bubônica na localidade de Jacocha, Huancabamba, Perú

As alterações climáticas produzidas no norte do Perú devidas ao Fenômeno El Niño (ENSO), ocasionaram variações no volume das safras, redistribuição do curso dos rios e provavelmente aumento da população de roedores. Em fevereiro de 1999, em uma comunidade indígena em Jacocha, Huancabamba, na serra de Piura, Perú, surgiu um surto de peste com cinco casos humanos, um dos quais faleceu. O diagnóstico foi confirmado pela sorologia (hemaglutinação passiva). A presença de anticorpos em cães de localidades próximas de Jacocha confirmaram a circulação da Yersinia pestis na área. O surto foi debelado pela rápida atuação das autoridades sanitárias locais. O episódio após silêncio epidemiológico por mais de quatro anos, mostrou a necessidade de reforçar o sistema de vigilância epidemiológica de peste nesta área.

Dermatite epidêmica por Paederus irritans em Piura, Perú, 1999, relacionada ao fenômeno El Niño

O fenômeno El Niño, que afetou o Perú em 1998, permitiu o crescimento de abundante vegetação em ambientes tradicionalmente secos. Paederus irritans, coleóptero que produz dermatite ao ser esfregado na pele, encontrou aí um substrato muito favorável ao incremento de sua população. A chegada das chuvas nos meses de fevereiro a abril de 1999 interferiu no hábitat desse inseto, que migrou inclusive para áreas urbanas, condicionando maior exposição da população humana de Piura, no norte do país, ao contato com esse agente. Entre fevereiro e maio de 1999 foram notificados em Piura 1.451 casos da dermatite por Paederus irritans, aí denominada latigazo dado o aspecto característico das lesões, eritematosas, lineares, semelhantes a chicotadas, por ele produzidas na pele por ação da pederina. As áreas do corpo mais afetadas foram a cabeça (56,6%) e o pescoço (30,9%), mas houve casos de conjuntivite e até de lesões genitais devidas à contaminação pelas mãos.

Von der Idee: Kultur

Im Werk Portugal Hoje. O Medo de Existir - Portugal heute. Die Angst des Existierens, zitiert F. Gil ein Lissabonner Graffitti. Não há drama, tudo é intriga." [[Kein Drama. Es ist alles Intrige], um daraus ein Motto für seine Analyse der gegenwãrtigen portugiesischen Gesellschaft zu entnechmen. Unfãhig, ihre herkõmmlichen Formen von Solidaritãt und gegenseitiger Hilfe wieder in Kraft zu stzen, sei die portugiesische Gesellschaft im chaotischen Ozean der Konkurrenz, der Arbeitslosigkeit und des Ausschlusses so tief gesunken, dass sie sich wegen der ihr gestellten Herausforderungen bedroht fühle. Der portugiesischen Gesellschaft würde es nicht gelingen, die Angst zu überwinden, die sie direkt aus der ehemaligen autoritãren und diktatorialen Gesellschaft als Erbe bekommen hatte, deren strukturelle Hauptstütze die Angst war - wie auch ein System, dessen Hauptziel es war, die Fõrderung der Kreativitãt , der schõpferischen und unternehmerischen Krãfte des sozialen, wirtschaftlichen Lebens zu unterdrücken; unter der Diktatur Salazars lebte man schon unter dem Einfluss der Gehorsamkeit, der Korruption, des Nicht-Teilnehmens, des Nicht-Einwerbens, des Nicht-Ereignens.

Nosocomial infections in a Brazilian neonatal intensive care unit: a 4-year surveillance study

INTRODUCTION: Report the incidence of nosocomial infections, causative microorganisms, risk factors associated with and antimicrobial susceptibility pattern in the NICU of the Uberlândia University Hospital. METHODS: Data were collected through the National Healthcare Safety Network surveillance from January 2006 to December 2009. The patients were followed five times/week from their birth to their discharge or death. RESULTS: The study included 1,443 patients, 209 of these developed NIs, totaling 293 NI episodes, principally bloodstream infections (203; 69.3%) and conjunctivitis (52; 17.7%). Device-associated infection rates were as follows: 17.3 primary bloodstream infections per 1,000 central line-days and 3.2 pneumonias per 1000 ventilator-days. The mortality rate in neonates with NI was 11.9%. Mechanical ventilation, total parenteral nutrition, orogastric tube, previous antibiotic therapy, use of CVC and birth weight of 751-1,000g appeared to be associated with a significantly higher risk of NI (p < 0.05). In multiple logistic regression analysis for NI, mechanical ventilation and the use of CVC were independent risk factors (p < 0.05). Coagulase- negative Staphylococcus (CoNS) (36.5%) and Staphylococcus aureus (23.6%) were the most common etiologic agents isolated from cultures. The incidences of oxacillin-resistant CoNS and S. aureus were 81.8% and 25.3%, respectively. CONCLUSIONS: Frequent surveillance was very important to evaluate the association of these well-known risk factors with NIs and causative organisms, assisting in drawing the attention of health care professionals to this potent cause of morbidity.

Reduction of catheter-associated bloodstream infections through procedures in newborn babies admitted in a university hospital intensive care unit in Brazil

INTRODUCTION: Catheter-associated bloodstream infection (CA-BSI) is the most common nosocomial infection in neonatal intensive care units. There is evidence that care bundles to reduce CA-BSI are effective in the adult literature. The aim of this study was to reduce CA-BSI in a Brazilian neonatal intensive care unit by means of a care bundle including few strategies or procedures of prevention and control of these infections. METHODS: An intervention designed to reduce CA-BSI with five evidence-based procedures was conducted. RESULTS: A total of sixty-seven (26.7%) CA-BSIs were observed. There were 46 (32%) episodes of culture-proven sepsis in group preintervention (24.1 per 1,000 catheter days [CVC days]). Neonates in the group after implementation of the intervention had 21 (19.6%) episodes of CA-BSI (14.9 per 1,000 CVC days). The incidence of CA-BSI decreased significantly after the intervention from the group preintervention and postintervention (32% to 19.6%, 24.1 per 1,000 CVC days to 14.9 per 1,000 CVC days, p=0.04). In the multiple logistic regression analysis, the use of more than 3 antibiotics and length of stay >8 days were independent risk factors for BSI. CONCLUSIONS: A stepwise introduction of evidence-based intervention and intensive and continuous education of all healthcare workers are effective in reducing CA-BSI.

Ecological aspects of mosquitoes (Diptera: Culicidae) in the gallery forest of Brasilia National Park, Brazil, with an emphasis on potential vectors of yellow fever

Introduction We analyzed the vertical and monthly distributions of culicid species in the gallery forest of Brasília National Park, with an emphasis on the potential vectors of yellow fever (YF). Methods Between September 2010 and August 2011, mosquitoes were captured on the ground and in the canopy of the forest for five consecutive days per month, from nine to 15 hours. The mosquitoes were examined to verify natural infection with flaviviruses by isolation in Aedes albopictus Skuse, 1864 cells followed by indirect immunofluorescence. Results We identified 2,677 culicids distributed in 29 species. Most of the mosquitoes were captured at ground level (69%) during the rainy season (86%). The most abundant species were Sabethes (Sabethes) albiprivus Theobald, 1903; Limatus durhamii Theobald, 1901; Haemagogus (Conopostegus) leucocelaenus Dyar & Shannon, 1924; Haemagogus (Haemagogus) janthinomys Dyar, 1921; Aedes (Ochlerotatus) scapularis Rondani, 1848; Psorophora (Janthinosoma) ferox Von Humboldt, 1819; and Aedes (Ochlerotatus) serratus Theobald, 1901. Limatus durhamii, Limatus durhamii, Psorophora ferox, Aedes scapularis and Aedes serratus showed significant differences (p<0.05) in their habitat use. Limatus durhamii was found more often in the canopy, unlike the other species. During the rainy season, the most abundant species were Sa. albiprivus, Haemagogus leucocelaenus and Limatus durhamii. During the dry season, the potential YF vectors exhibited a very low frequency and abundance, except Aedes scapularis and Aedes serratus. No flavivirus was detected in the 2,677 examined mosquitoes. Conclusions We recommend continued and systematic entomological monitoring in areas vulnerable to the transmission of YF in the Federal District of Brazil.

Nosocomial infections in a neonatal intensive care unit during 16 years: 1997-2012

Introduction Surveillance of nosocomial infections (NIs) is an essential part of quality patient care; however, there are few reports of National Healthcare Safety Network (NHSN) surveillance in neonatal intensive care units (NICUs) and none in developing countries. The purpose of this study was to report the incidence of NIs, causative organisms, and antimicrobial susceptibility patterns in a large cohort of neonates admitted to the NICU during a 16-year period. Methods The patients were followed 5 times per week from birth to discharge or death, and epidemiological surveillance was conducted according to the NHSN. Results From January 1997 to December 2012, 4,615 neonates, representing 62,412 patient-days, were admitted to the NICU. The device-associated infection rates were as follows: 17.3 primary bloodstream infections per 1,000 central line-days and 3.2 pneumonia infections per 1,000 ventilator-days. A total of 1,182 microorganisms were isolated from sterile body site cultures in 902 neonates. Coagulase-negative staphylococci (CoNS) (34.3%) and Staphylococcus aureus (15.6%) were the most common etiologic agents isolated from cultures. The incidences of oxacillin-resistant CoNS and Staphylococcus aureus were 86.4% and 28.3%, respectively. Conclusions The most important NI remains bloodstream infection with staphylococci as the predominant pathogens, observed at much higher rates than those reported in the literature. Multiresistant microorganisms, especially oxacillin-resistant staphylococci and gram-negative bacilli resistant to cephalosporin were frequently found. Furthermore, by promoting strict hygiene measures and meticulous care of the infected infants, the process itself of evaluating the causative organisms was valuable.

Real convergence in per capita output and growth in Eurozone: A time-series approach

The real convergence hypothesis has spurred a myriad of empirical tests and approaches in the economic literature. This Work Project intends to test for real output and growth convergence in all N(N-1)/2 possible pairs of output and output growth gaps of 14 Eurozone countries. This paper follows a time-series approach, as it tests for the presence of unit roots and persistence changes in the above mentioned pairs of output gaps, as well as for the existence of growth convergence with autoregressive models. Overall, significantly greater evidence has been found to support growth convergence rather than output convergence in our sample.

Inventare musei per ordinare e rappresentare il mondo. La Guardaroba nuova di Palazzo Vecchio e le Sale della cosmografia e delle matematiche agli Uffizi a Firenze

Este artigo traça a história da invenção e construção de dois lugares museológicos (um literário e outro real) na Florença da segunda metade do século XVI, chamando a atenção para o seu significado no contexto do saber da época. O projecto cosmográfico para o Guarda-roupa novo do Palazzo Vecchio e as Salas da Cosmografia e das Matemáticas nos Uffizi oferecem a possibilidade de explorar dois sistemas semióticos que permitem explorar os processos de invenção e criação do museu como lugar – literário, imaginário, arquitectónico, epistemológico – no qual e através do qual se ordena e representa o mundo. O primeiro destes espaços é o projecto cosmográfico idealizado por volta de 1560 por Cosimo I, Giorgio Vasari e Miniato Pitti para o Guarda-roupa novo do Palazzo Vecchio, actualmente conhecido, impropriamente, como “Sala das cartas geográficas”. O projecto nunca foi concluído; porém existiu e continua a existir e a fascinar como “espaço literário” através de uma página visionária na segunda edição das Vite de Giorgio Vasari. O segundo lugar é a Sala da Cosmografia, mandada construir por Ferdinando I em 1589, juntamente com a contígua Sala das Matemáticas o da Arquitectura Militar na Galleria degli Uffizi. No centro do novo projecto expositivo, totalmente concluído, pela primeira vez, foram colocados os instrumentos e livros científicos e, implicitamente, o Homem como observador e demiurgo do mundo.

“Del palazzo de’ cesari” genesi e riferimenti dell’attività di Filippo Juvarra per D. João V di Portogallo

Tal como os outros soberanos do Antigo Regime, D. João V procurava a legitimidade do Império Português a nível europeu, através dos grandes projetos arquitetónicos. O novo palácio real e a igreja patriarcal que Juavarra desenhou em 1719 para a cidade de Lisboa teve que representar e simbolizar o poder do rei. Um estudo pormenorizado sobre parte da arquitectura de Juvarra parece revelar uma estreita relação, não com a contemporânea Roma dos Papas, mas antes com a majestosa arquitectura da Roma Imperial. D. João V tinha o ensejo de ser considerado um imperador e a arquitectura de Juvarra demonstra-o claramente.